D-glucosyl-N-acylsphingosine

Three steps

• (11Z,14Z)-eicosadienoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (17Z)-hexacosenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (7Z,10Z,13Z,16Z)-docosatetraenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• a 1,3-diacyl-sn-glycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 3-acyl-sn-glycerol + an acyl-CoA = a 1,3-diacyl-sn-glycerol + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatusOne of 3 routes

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphocholine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatus

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatus

• a fatty acyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  3. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate

  Catalysed by GBA1, GBA3, GBA2, NAAA and 6 morecytosol

• N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatusOne of 3 routes

• (5Z,8Z,11Z)-eicosatrienoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerol + a sphingomyelin = a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 1 morecytosol, Golgi apparatusOne of 2 routes

• a ganglioside GD3 (d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 33 moreendoplasmic reticulum, cytosolOne of 4 routes

• a ganglioside GM4 (d18:1(4E))Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP-N-acetyl-beta-neuraminate = a ganglioside GM4 (d18:1(4E)) + CMP + H(+)

  Catalysed by GBA1, GBA3, GBA2, UGT8 and 1 morecytosol

• tricosanoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• cholesteryl octadecanoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. octadecanoyl-CoA + cholesterol = cholesteryl octadecanoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT1cytosol

• cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT2 and 1 morecytosolOne of 2 routes

• sphinga-4E,14Z-dienineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatus

• beta-D-glucosyl-(1<->1)-sphing-4-enineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. an N-acylsphing-4-enine + UDP-alpha-D-glucose = a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + H2O = beta-D-glucosyl-(1<->1)-sphing-4-enine + a fatty acid

  Catalysed by GBA1, GBA3, GBA2, UGCG and 1 morecytosol

• cholesteryl (9Z)-hexadecenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (9Z)-hexadecenoyl-CoA + cholesterol = cholesteryl (9Z)-hexadecenoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT1cytosol

• (9Z,12Z,15Z-octadecatrienoyl)-cholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + cholesterol = (9Z,12Z,15Z-octadecatrienoyl)-cholesterol + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT2cytosolOne of 2 routes

• cholesteryl (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 1-hexadecanoyl-2-(8Z,11Z,14Z-eicosatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (8Z,11Z,14Z)-eicosatrienoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GBA1, GBA3, GBA2, LCATcytosol

• (5Z)-tetradecenoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + cholesterol = (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterol + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT2cytosolOne of 2 routes

• 4beta-hydroxycholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 4beta-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA3, GBA2, CYP3A4cytosol

• an N(2)-acyl-L-glutamineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatus

• (7Z)-hexadecenoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• cholesteryl (5Z,8Z,11Z)-eicosatrienoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z-eicosatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (5Z,8Z,11Z)-eicosatrienoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GBA1, GBA3, GBA2, LCATcytosol

• (6Z,9Z,12Z-octadecatrienoyl)-cholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 1-hexadecanoyl-2-(6Z,9Z,12Z-octadecatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = (6Z,9Z,12Z-octadecatrienoyl)-cholesterol + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GBA1, GBA3, GBA2, LCATcytosol

• cholesteryl (11Z)-octadecenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (11Z)-octadecenoyl-CoA + cholesterol = cholesteryl (11Z)-octadecenoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT1cytosol

• beta-D-galactosyl-(1->4)-[alpha-L-fucosyl-(1->3)]-D-glucoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose + UDP-alpha-D-galactose = lactose + UDP + H(+)
  3. lactose + GDP-beta-L-fucose = beta-D-galactosyl-(1->4)-[alpha-L-fucosyl-(1->3)]-D-glucose + GDP + H(+)

  Catalysed by GBA1, GBA3, GBA2, B4GALT2 and 3 morecytosol

• tricosanoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• a 3-oxoacyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + acetyl-CoA = a 3-oxoacyl-CoA + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 7 morecytosol, Golgi apparatus